ICHD 3 – Guide to use and key definitions ASHS UCNS Board Review Morris Levin Chief, Division of Headache Medicine Department of Neurology, UCSF Disclosures for Dr. Morris Levin Consultant: Supernus, Upsher-Smith, Lilly, Amgen, Allergan, Alder, Teva Royalties: Oxford University Press, Anadem Publishing, Wiley Blackwell, Castle Connolly Publishing, UCSF Office of Innovation History • International classification of headache disorder , 1st edition (ICHD-1) in 1988 • ICHD-2 in 2004 • ICHD-3 beta in 2013 • ICHD-3 in 2018* *Based on 4 years of input including field testing and research aimed at ICHD 3 increasing precision and accuracy Painful structures in the head (neck) • Dura • Dural veins and arteries • Intracran arteries • V,VII,IX,X,C1-3 • Periosteum • Scalp • Scalp muscles • Scalp vessels • Sinuses • Eyes • Ears • Teeth, TMJ • Carotids, vertebrals • C-spine periost • Cerv musc Painful structures in the head (neck) Not painful • Parenchyma • Pia, arachnoid • Skull • Ventricles ICHD III – Basic Organization Part 1: Primary headaches Part 2: Secondary headaches Part 3: Cranial Neuralgias, etc. The Appendix ICHD III The Appendix: 1. Suggested criteria for possible new entities 2. Alternative diagnostic criteria for certain categories (pending evidence) 3. Some previously accepted disorders which have not been supported by evidence ICHD III Part 1: Primary headaches (no other causative disorder) 1. Migraine 2. Tension-type Headache 3. Cluster and its relatives (TACs) 4. Other primary headaches – exertional, hypnic headache, NDPH, et al Migraine 1.1 Migraine without aura Diagnostic criteria: A. At least five attacks1 fulfilling criteria B–D B. Headache attacks lasting 4–72 hours (when untreated or unsuccessfully treated) C. Headache has at least two of the following four characteristics: 1. unilateral location 2. pulsating quality 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity D. During headache at least one of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia 1.2 Migraine with Aura ICHD 3 b A. At least two attacks fulfilling criteria B and C B. One or more of the following fully reversible aura symptoms: 1. visual 2. sensory 3. speech and/or language 4. motor 5. brainstem 6. retinal C. At least 2 of the following 4 characteristics: 1. at least one aura symptom spreads gradually over ≥5 minutes and/or two or more aura symptoms occur in succession 2. each individual aura symptom lasts 5-60 minutes 3. at least one aura symptom is unilateral 4. the aura is accompanied, or followed within 60 minutes, by HA D. Not better accounted for by another ICHD-3 diagnosis. New Definition of 1.2 Migraine with Aura ICHD 3 Final A. At least two attacks fulfilling criteria B and C B. One or more of the following fully reversible aura symptoms: 1. visual 2. sensory 3. speech and/or language 4. motor 5. brainstem 6. retinal C. At least three of the following six characteristics: 1. at least one aura symptom spreads gradually over ≥5 minutes 2. two or more aura symptoms occur in succession 3. each individual aura symptom lasts 5-60 minutes 4. at least one aura symptom is unilateral 5. at least one aura symptom is positive 6. the aura is accompanied, or followed within 60 minutes, by headache D. Not better accounted for by another ICHD-3 diagnosis. New Definition of Migraine with Aura “…(new) 1.2 criteria performed much better in distinguishing MA from TIA ICHD is dynamic 1.2 Migraine with typical aura A. Attacks fulfilling criteria for 1.2 Migraine with aura and criterion B below B. Aura with both of the following: 1. fully reversible visual, sensory and/or speech/ language symptoms 2. no motor, brainstem or retinal symptoms. Migraine with Aura ICHD III With headache Migraine with typical aura Without Migraine with headaches aura Migraine with brainstem aura Hemiplegic Migraine Retinal migraine 1.2.4 Retinal migraine A. At least 2 attacks fulfilling criteria B and C B. Aura of fully reversible monocular positive and/or negative visual phenomena confirmed during an attack by either or both of the following: 1. clinical visual field examination 2. patient’s drawing of a monocular field defect C. ≥2 of the following 3 characteristics: 1. aura spreads gradually over ≥5 min 2. aura symptoms last 5-60 min 3. aura accompanied or followed in <60 min by headache Retinal migraine v. Binocular aura The typical migraine visual aura is a binocular visual disturbance. Most patients describe the disturbance as monocular as it tends to occur in either the right or left visual field, which they interpret as occurring in the eye on the side of the disturbance. It is present with eyes open or closed. Careful questioning almost always reveals that it occurs as a binocular phenomenon. Retinal migraine v. Binocular aura How to tell if symptoms are emanating from one eye or are homonymous? If positive phenomena are emanating from one retina e.g. – closing that eye will NOT eliminate them If negative phenomena are emanating from one retina, closing that eye will eliminate them. But negative phenomena emanating from one retina may not have been noticed in the first place if that is not the dominant eye. 1.2.4 Retinal migraine A. At least 2 attacks fulfilling criteria B and C B. Aura of fully reversible monocular positive and/or negative visual phenomena confirmed during an attack by either or both of the following: 1. clinical visual field examination 2. patient’s drawing of a monocular field defect C. ≥2 of the following 3 characteristics: 1. aura spreads gradually over ≥5 min 2. aura symptoms last 5-60 min 3. aura accompanied or followed in <60 min by headache D. Not better accounted for by another ICHD-3 diagnosis, and other causes of amaurosis fugax excluded 1.2.2 Migraine with brainstem aura A. At least 2 attacks fulfilling criteria B and C below, and criteria C and D for 1.2.1 Migraine with typical aura B. Aura of fully reversible visual, sensory and/or speech/language symptoms, but not motor or retinal C. 2 of the following brainstem symptoms: 1. dysarthria 2. vertigo 3. tinnitus 4. hypacusis 5. diplopia 6. ataxia 7. decr level of consc Richter, F et al. Enhanced neuronal excitability in adult rat brainstem causes widespread repetitive brainstem depolarizations. Jnl Cereb Blood Flow Metab 2012; 32:1535-45. Noseda, R, & Burstein, R (2013). Migraine pathophysiology: anatomy of the trigeminovascular pathway and associated neurological symptoms, cortical spreading depression, sensitization, and modulation of pain. PAIN 2013, 154, S44-S53. – “descendent modulation of the brainstem trigeminovasc system” from CSD projected to brainstem via descending neuronal pathways 1.2.2 Migraine with brainstem aura A. At least 2 attacks fulfilling criteria B and C below, and criteria C and D for 1.2.1 Migraine with typical aura B. Aura of fully reversible visual, sensory and/or speech/language symptoms, but not motor or retinal C. 2 of the following brainstem symptoms: 1. dysarthria 2. vertigo 3. tinnitus 4. hypacusis 5. diplopia 6. ataxia 7. decr level of consc Richter, F et al. Enhanced neuronal excitability in adult rat brainstem causes widespread repetitive brainstem depolarizations. Jnl Cereb Blood Flow Metab 2012; 32:1535-45. Noseda, R, & Burstein, R (2013). Migraine pathophysiology: anatomy of the trigeminovascular pathway and associated neurological symptoms, cortical spreading depression, sensitization, and modulation of pain. PAIN 2013, 154, S44-S53. – “descendent modulation of the brainstem trigeminovasc system” from CSD projected to brainstem via descending neuronal pathways A1.6.5 Vestibular migraine – a proposed migraine subtype A. At least five episodes fulfilling criteria C and D B. A current or past history of 1.1 Migraine without aura or 1.2 Migraine with aura1 C. Vestibular symptoms of moderate or severe intensity, lasting between 5 minutes and 72 hours D. At least 50% of episodes are associated with at least one of the following three migrainous features: 1. headache with at least two of the following four: a)unilat location b) pulsating quality c) moderate or severe intensity d) aggravation by routine activity 2. photophobia and phonophobia 3. visual aura Migraine with Aura ICHD III With headache Migraine with typical aura Without headaches Migraine with Migraine with aura brainstem aura Migraine with hemiplegia Retinal migraine Vestibular migraine Menstrual migraine is not listed in the ICHD3 – considered a trigger – But: Appendix • A1.1.1 Pure menst mig without aura • A1.1.2 Menstrually related mig without aura • A1.1.3 Non-menst mig without aura Chronic migraine defined as migraine or strongly suspected migraine on >15d/mo ICHD3 1.3 Chronic Migraine A. Headache on ≥15 d/mo for >3 mo B. In a patient who has had ≥5 attacks fulfilling criteria for 1.1 Migraine without aura or 1.2 Migraine with aura C. On ≥8 d/mo for >3 mo fulfilling any of the following: 1. criteria C and D for 1.1 Migraine without aura 2. criteria B and C for 1.2 Migraine with aura 3. believed by the patient to be migraine at onset and relieved by a triptan or ergot derivative D. Not better accounted for by another ICHD-3 diagnosis Imaging studies support the distinction between EM and CM Maniyar, FH et al. Functional imaging in Chronic Migraine. Curr Headache and Pain Reports, 2013. Increasing frequency of migraine attacks is associated with changes in key brainstem areas, basal ganglia and various cortical areas involved in pain. Schwedt, T, et al Headache 2015 Accurate subclassification of individuals into lower and higher frequency subgroups via measurements of cortical thickness (and other measurements in temporal pole, anterior cingulate cortex, superior temporal lobe, entorhinal cortex, medial orbital frontal gyrus, and pars triangularis.